Thursday, October 10, 2013

Good news, everyone!

In the immortal words of Futurama's Professor Hubert Farnsworth, who I am growing to look like more every day, "Good news, everyone!"

Since my last post I've gotten some encouraging results from a PET/CT scan, and I've passed three dates of note on the calendar.

First, the PET/CT, which was done as I was finishing the fourth round of R-EPCH in early September. A PET/CT is how doctors keep track of the progress being made by DLBCL patients (remember, thanks to Richter's Transformation some of my CLL became Diffuse Large B Cell Lymphoma).

My first PET/CT in April, at diagnosis, was scary. It showed massive tumors, "too numerous to count," which lit up like a Christmas tree. When I say "lit up," I mean that the standard uptake value (SUV) of the radioactive glucose solution they put in my bloodstream was positive for aggressive lymphoma. (Oh how I wish I lived in a world where the only thing SUV meant to me was "sport utility vehicle")

The SUV of the tumors ranged from a high of 26.20 down to 14.03. By comparison, "background" SUV -- that of noncancerous normal tissues, is 2.5 in the liver and 1.5 to 2.0 in the mediastinal blood pool. Both are often used as comparison tools. According to one research paper:

a SUV ≥ 13 in the most intense lesion is highly indicative of
aggressive histology, while a SUV ≤ 6 is much more compatible with indolent lymphoma, unless
the clinical course indicates otherwise

My September scan showed a marked reduction in swollen lymph nodes, with few remaining. And the SUV of the node under my left arm, for example, dropped from 17.2 in April to 1.1 in September. The largest nodal mass in my abdomen showed an SUV drop from 18.8 to 1.8 (and a drop in size from 25 x 25 cm in April to 13.4 x 9.2).

In other words, my SUV was now at background levels. My oncologist, Dr. Droll, said, "If there were still high-grade lymphoma, it wouldn't look like this. . . . These things light up like CLL would light up."

Needless to say, Marilyn and I were quite happy with the report, which was better than we dared to hope.

Does that mean I'm lymphoma-free, back to plain old CLL, which is, I hate to say it, by comparison a "good" cancer, or at least much better-behaved?

Well, maybe. I have learned that a CR ("complete response") in DLBCL is very much like a CR in CLL: there may still be some cancer cells lurking around that still may cause a relapse, not all of which are detectable on even the most sophisticated test. As with CLL, clinical symptoms are the bottom line, and only time will tell if I relapse.

I still have some swollen nodes and an enlarged spleen. Maybe they're all filled with CLL, and maybe there are just a few really nasty lymphoma cells that have survived and are lurking in there somewhere.

Does my response to the chemo mean that I can put off having a transplant? Possibly. But there is still wisdom in considering the transplant option. CLL doesn't get better the longer you have it, and neither, I presume, does DLBCL. (As one lymphoma expert pointed out to me, the CLL could always transform again.) At the age of 57 I am still young enough to tolerate a transplant -- I have tolerated the chemo quite well -- but I won't be a picture of youth and vigor forever.

Further, to get me to this point I have undergone some heavy-duty chemo that I can't responsibly use again, even as transplant conditioning. Adriamycin is a drug that does wonders against lymphoma, and it is a component of both R-EPOCH and R-CHOP. It has also been implicated in congestive heart failure if you do too much of it, and if I were to do any more past Round 6 of R-EPCH, I would be doing too much.

Speaking of chemo, it is the standard of care for DLBCL to go the distance, all six rounds, of R-EPCH, even if the PET/CT after the fourth shows no evidence of lymphoma. The theory is that the more chemo, the more undetectable stuff you kill, the better off you are. So I have now completed Round 5 and I'm scheduled to go back to the hospital for the final round next week.

We've spent more than 40 days in the hospital this year -- I say "we" because Marilyn comes with me and sleeps in a fold-out chair next to me; hospitals really need double beds. The chemo is given over five days, with a visit to the doctor's office for a Neulasta shot on the sixth.

The hospital staff -- the chemo nurses of Six South at Banner Baywood Medical Center in Mesa, AZ -- have been great; I spent my birthday in there during the last round, and they surprised me with a round of "Happy Birthday" and a gift. Food service even provided a small cake.

The birthday is one of the calendar dates I mentioned at the start of this post. Another is the September 3 anniversary of my CLL diagnosis -- my tenth anniversary, as it turns out. No singing, no cake, just an "ugh" in recognition of the event.

The last date of note was October 1, the opening of the Health Insurance Marketplace. Thanks to President Obama and the Democrats, I will be able to purchase health insurance that covers a transplant. My current health plan specifically prohibits it, and because of my preexisting conditions, no one else would insure me -- until now, when preexisting conditions cannot be held against you.

So, bottom line, the Affordable Care Act is potentially a matter of life and death for me. Thank you, Mr. President. May we, as a nation, always listen to the better angels of our nature.

I've written about a new technology called "CAR T-cells" for CLL. CAR is short for "chimeric antigen receptor." In short, it is a new method of harnessing the power of your own immune system by reprogramming it to go after the cancer. Please see my prior posts for more complete description and amazingly cool video.

While it may sound like science fiction, it made quite a splash two years ago in the scientific literature and is now getting out to a bunch of universities. If I am not missing anyone, I think 10 separate academic programs currently have CAR T-cell programs going and Novartis has made a big push into the field as well.

Most of the programs out there have gone after CLL or Acute Lymphoblastic Leukemia (really super bad type of blood cancer). I wanted to highlight one program however that is taking the program and going after lymphoma. It is currently run out of the NIH.

I am honestly not sure how the current "government shutdown" is affecting clinical trial accrual for these studies but I think they are currently accruing patients with relapsed / refractory DLBCL and possibly even follicular lymphoma.

With NIH studies, you pay your way to the first visit, but if you get accepted into the trial, they cover all you subsequent travel expenses etc.

If I had relapsed DLBCL or multiply recurrent indolent lymphoma, I would be on the first plane to NIH for these studies. I thought I should call your attention to them. For more information about the study click here

ME AND MY DIARY

February 2014 in Sedona, AZ, slimmed down to 144 lbs.

My name is David Arenson and I have chronic lymphocytic leukemia. It may kill me. Then again, it may not. Life is full of surprises, although I must admit that this is not the sort of cliffhanger that I had in mind for my 50s.

Until a few years ago, like most people, I had assumed death and disease were the province of old age, not the prime of life. I was just an average person health-wise, and feeling rather fine, thank you. I passed by the occasional wheelchair-bound person or bald-headed chemotherapy patient and didn't think that sort of thing would ever apply to me. The odds were against it, after all. Then, after a blood test at age 46, I became one of those people.

And so, my life has changed. I still enjoy the same things I always have – my beautiful and wonderful soulmate, Marilyn, and music, and walks in the woods, and cheap Asian food at strip malls, and movies in which a giant reptile threatens an entire city.

But I also have a new reality that intrudes, one where mutant B lymphocytes threaten my entire body, and one which requires becoming accustomed to unfamiliar and intimidating territory. My spleen and lymph nodes are swollen and my neck sometimes looks like that of a chipmunk storing too many nuts; bothersome nodes in my left pelvic area are a constant reminder that something is wrong with my body. Over time my immunity has been degraded and I have had to rely more on antibiotics to shake infections that once gave me no pause. I have also experienced the joys of autoimmune hemolytic anemia, of which there are none, which is a scary condition in which the body destroys its own red blood cells, and which leads to fatigue.

My CLL has had more than a physical impact. It has been quite an education -- both in terms of what I have learned about my ability to cope with what once was unthinkable, and in terms of navigating the almost freakishly contradictory world of CLL management and treatment. Needless to say, only a fool treads there without getting the lay of the land; too many local doctors are simply clueless, and even the experts can disagree. I do not claim to have it all figured out, and I expect that I never will, but I am doing my best, and I hope some of my thoughts can be of use to you.

So, if sharing my journey helps you along the way, it will have been my pleasure, something green and growing in this hard, new landscape. We help each other as we can, and this is why we have a vibrant CLL community of websites, forums, and blogs (see links below). The end of the circle is the start of the circle. What goes around comes around.

BLOGGER PROFILE

Writing has been in my blood longer than CLL. I am a former newspaper reporter and editor and co-author with Marilyn of two humor-trivia books, Disco Nixon and Rambo Reagan. Marilyn and I met at the University of California at Santa Cruz and now live in the red rock country of Northern Arizona . . . CLL Diary has been featured in CR, the magazine of the American Association for Cancer Research, and in Family Practice Management, a publication of the American Academy of Family Physicians. Besides writing about CLL, I helped establish CLL Forum, one of the largest discussion groups for patients and caregivers.

CLL PROGNOSTICS & PLANNING

As we patients eventually learn, CLL is not a one-size-fits-all disease. Some cases are indolent, some progressive, some quite aggressive. Prognostic tests can give us a much better idea of what type of CLL we are dealing with. Knowledge is power, and I believe patients should have these tests and know what they mean. They do not provide a complete picture, and sometimes clinical symptoms tell a different story than one might expect from the results, but they are important tools that can help determine the when and what of treatment.

Here are the tests: IgVH mutational status, FISH, ZAP-70 (as done at a research institution such as UC San Diego, not a commercial lab), and CD38.

My tests indicate a progressing disease. I am IgVH unmutated and ZAP-70 positive, as measured at UCSD. I developed an 11q deletion per FISH in 2006, which disappeared in 2012 for some mysterious reason, giving way to a 13q deletion. I am CD38 positive now, despite having been CD38 negative for years.

Given my tender age, I will always be navigating treatment options if I want to have any hope of living a normal life span. Knowing my test results helps me plan ahead, and knowing the possible end point in my battle with CLL helps me plan what treatments make the most sense, and in what order. Like many CLLers, I am encouraged by the progress being made by new drugs such Ibrutinib and ABT-199; not to mention the news that T-cells can be supercharged to wipe out the CLL -- in much the same ferocious way that macrophages went after my red cells during hemolysis with AIHA.

The "when and what" of treatment is a subject of great debate among CLL experts as well as patients and local doctors. I tend to take a conservative approach, ever aware of the fact that overall survival in CLL depends not just on the effectiveness of your first treatment. What you do for an encore -- your ability to respond to treatment again, and then again -- may determine how long you get to stand on the stage. The late CLL expert Dr. Terry Hamblin once wrote that CLL is a war of attrition, and I am ever mindful that such wars are won, if they can be won, slowly.

Whether my decisions ultimately are proved wise will be written in these pages. I began using single-agent rituximab (Rituxan) in 2004, adding the steroid methylprednisolone in March 2007 to combat AIHA. In October 2007, after a severe AIHA relapse that left me steroid refractory, I was treated with Rituxan + cyclophosphamide, vincristine, and prednsione (R-CVP). In January 2009, when AIHA and hemolysis of red blood cells returned, I had Rituxan + cyclophosphamide and dexamethasone (R-CD). I used this a few times to control the condition, with shorter and shorter periods until AIHA relapse. Starting in February 2010 I used Arzerra (ofatumumab) and Revlimid (lenalidomide), and then for a year and a half maintained control of the disease -- and the AIHA -- with Revlimid alone. Alas, the Revlimid came at a high price in terms of blood clotting issues, and as of 2012 I was treated with bendamustine and rituximab, which gave me a CR in the marrow and blood, leaving some swollen lymph nodes behind.

2013 is turning out to be my most challenging year yet, with the arrival of Richter's Transformation in April. Up to 10% of CLL patients can expect to develop Richter's, in which some of the CLL clones mutate into a more dangerous B cell lymphoma. Richter's is fatal in some 50% of cases, but it also can be beaten with chemotherapy and stem cell transplant. Read my latest posts for updates on my experience.

My best advice to patients is to gather all the facts you can about your CLL and then think ahead and plan ahead. Develop a long-term strategy, but expect to have to roll with the punches. And don't be rushed by doctors, family, or anyone else into a decision you are not comfortable with: Treating CLL is almost never an emergency. Take the time to learn and reflect, and then go with your intuition.

There are no guarantees that your choices will work out, of course, but at least you can rest assured that you put your heart and soul into making them. That sort of effort is the effort that can, with luck, beat cancer.

It's a peace sign, or a V for victory, not sure which

Quotes I Like

"The thing in life is not to know all the answers but rather to ask the right questions." -- Anonymous

"Hope is not the conviction that something will turn out well, but the certainty that something makes sense, regardless of how it turns out." -- Vaclav Havel

"The man who never alters his opinion is like standing water, and breeds reptiles of the mind." -- Blake

"We must be willing to let go of the life we have planned so as to have the life that is waiting for us." -- E.M. Forster

"Think of all the beauty still left around you and be happy." -- Anne Frank

“Panic is a projection that is not real. We are not just our fears. Our fears do not necessarily determine our future. This is significant.” -- Greg Anderson, lung cancer survivor

"I had a choice to make when they said I was going to die. I could chose to live the rest of my life dying, or I could chose to live life until I die. And I chose to live life'. -- Anonymous cancer patient

"Life can only be understood backwards; but it must be lived forwards." -- Soren Kierkegaard

A DISCLAIMER

I am not a doctor and I do not play one on the internet. If you take something I say as medical advice and die as a result, perhaps in your next life you will not believe everything you read on the internet.

Copyright 2005-2014 by David Arenson. All rights reserved. Material is for the personal use of CLL patients and caregivers and may not be used or reproduced for commercial purposes.